Facioscapulohumeralmusculardystrophy(FSHD)iscausedbythedeletionofasubsetofD4Z4macro-satelliterepeatsonchromosome4.Eachrepeatcontainsaretrogeneencodingthedouble-homeoboxfactorDUX4.DUX4expressionisepi-geneticallysuppressedindifferentiatedtissues,andtheresidualDUX4transcriptsaresplicedtoremovethecarboxyterminaldomainthathasbeenassociatedwithcelltoxicity.InFSHDindividuals,theexpressionofthefull-lengthDUX4transcriptisnotcompletelysuppressedinskeletalmuscleandpossIBLyotherdifferentiatedtissues.

ThisantibodyrecognizesanepitopeattheC-terminusofhumanDUX4.Itdoesnotcross-reactwithDUX4c.

SniderLetal2010PLoSGenetics6:1-14.

Forinvitroinvestigationaluseonly.Notintendedfordiagnosticortherapeuticapplications.

Immunohistochemistry:frozenorparaffin.

Immunofluorescence:

#57100diluted1:10,000onrhaBDomyosarcomacelllineRDtransfectedwithpCS2-DUX4.CellswerecounterstainedwithDAPIfornuclei.

Immunoblotting:

#57100diluted1:10,000onC2C12cellstransfectedwithpCS2-DUX4.Thereisadoubletbecausetheexpressionconstructcontainsanupstreamalternatetranslationstartcodon.ThelowerbandisthecanonicalsizeforDUX4.

Positivecontrol:humantestislysate.

DiluteinPBSormediumwhichisidenticaltothatusedintheassaysystem.

Thisantibodyisstableforatleastone(1)yearat-20oC.

美国QED Bioscience 公司（www.qedbio.com）是专业的抗体公司，有20年的历史，是世界著名的抗体生产商。QED生物科技为科研客户提供优质的单克隆和多克隆抗体产品。 是一家提供全面服务的并提供额外服务的公司，能提供超过1500的单克隆和多克隆抗体的用于多领域的研究。